Causes of ALS: Abnormal Mitochondria

Mitochondria are small bodies that act as the powerhouses of every cell. They supply energy crucial to cell survival.

It’s no secret that abnormal, poorly functioning or dying mitochondria are common in neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS). Unusual-appearing mitochondria appear early in animal models of ALS – even before disease symptoms surface.

Recent Packard research also shows that mitochondrial movement throughout motor neurons – a normal occurrence – becomes erratic. How this affects the availability of energy at key places in motor neurons, such as synapses, isn’t well understood. It’s a current topic of our Center’s grantees’ research.

Packard Center scientists have been actively pursuing possible biochemical causes of ailing, abnormal mitochondria in the SOD1 mouse model of familial ALS and in model yeasts, with the thought that understanding what’s happening to the tiny cell bodies will shed light on what starts the disease, both in familial and sporadic ALS.

Learn more about the research projects funded and coordinated by the Packard Center for ALS Research at Johns Hopkins, and targeted at finding the causes of ALS and a cure. Subscribe to ALS Alert to stay informed about ALS research and clinical trials.